Heidi Collins Fantasia Benign vulvar disorders, which encompass a broad range of dermatologic conditions, account for significant patient concern. Women may delay seeking treatment owing to fear, embarrassment, or confusion over which health care provider would best understand their condition. Therefore, symptoms may be present for some time before women seek care. Signs and symptoms of benign vulvar disorders can include pruritus, pain, burning, irritation, and a mass and/or growth. Women have often tried nonprescription remedies before coming to a provider.1 Pruritus is often the chief patient complaint for most vulvar disorders, and skin breakdown is common from scratching. Vulvar pruritus is a nonspecific vulvar symptom that may be unrelated to vaginitis, sexually transmitted infections (STIs), Bartholin duct cysts, or neoplasms. Proper treatment of vulvar pruritus depends on an accurate diagnosis. Women with vulvar pruritus often receive multiple treatments in the absence of a correct diagnosis; women are commonly prescribed therapy over the phone without ever having been examined, even if symptoms have been recurrent.2 In examining women with vulvar pruritus, the provider is advised to ask about and to inspect other areas of the body; many conditions affecting other organ systems, including tuberculosis, Crohn disease, and endometriosis, can have vulvar manifestations. For example, vulvar psoriasis may have an unusual presentation, but more typical psoriatic lesions are often seen simultaneously elsewhere on the body and may provide a diagnostic clue. Even with an experienced clinician, several visits may be needed to diagnose and to improve certain vulvovaginal conditions. The visual inspection is essential in identifying vulvar changes. A hand-held microscope, or in some cases a colposcope, may allow more detailed inspection. Vaginitis, cervicitis, and other STIs should be excluded. Some of the common vulvar conditions causing pruritus are presented in this chapter. Lichen sclerosus (LS) is a chronic condition with an unclear cause. Multiple factors are most likely involved in the development of this disorder. LS can have an autoimmune component, and women with LS may also develop other autoimmune diseases such as thyroid disorders, anemia, vitiligo, and alopecia areata. It has also been postulated that LS may be triggered by an infectious process, a genetic predisposition, and decreased estrogen levels.3 LS is primarily seen in perimenopausal and postmenopausal women, and the incidence increases with age. Although LS can occur in females of all ages, postmenopausal women are affected more frequently than women of other age groups. Approximately 1 in 30 older women has LS, and it is especially common in women with psoriasis.4 It also affects males but at rates much lower than in females. LS is primarily found in the anogenital region, but it can be seen elsewhere on the body, such as the neck and shoulders. Although it is sometimes asymptomatic, LS often results in severe vulvar pruritus and/or dyspareunia. Affected areas include the labia minora, vulvar vestibule, perineum, and clitoris; the vagina is usually spared. Early LS can be particularly difficult to diagnose. Early in the disease process, women may report vague vulvar burning and generalized irritation or itching that is difficult for them to localize and quantify. On examination, white papules can be seen, and the epithelium may appear normal or thin, resembling parchment.5 Typically, tissue elasticity is decreased and edema may be present, depending on the disease stage. Fissures and secondary infections may develop, especially with sexual activity or scratching, which may make diagnosis especially difficult. With disease progression, papules develop into large, hypopigmented, symmetric plaques, often hourglass or keyhole shaped, on the labia minora, vulva, and anal area, which can resemble hyperplasia.6 If these are not treated, there is eventual loss of vulvar architecture such that the labia minora are no longer seen, and introital stenosis may develop, resulting in dyspareunia, ecchymosis, fissures, and telangiectasis. • Thorough history is obtained to assess timing, onset, location, and duration of symptoms and any factors that may alleviate or aggravate the condition. • Fungal infections such as external yeast have a more rapid onset and are associated with erythema. • Physical examination is performed to visualize any skin changes. • May be performed with hand-held magnifying glass or colposcope to improve visualization of the skin. • Other diagnostics to consider include the following: • Complete blood count (CBC) if infection is suspected from scratching or skin breakdown. It is important to establish the correct diagnosis of LS. This condition requires long-term treatment. Women should be prepared for the chronicity of the disorder and expected length of time to symptom improvement. The primary goals of treatment are symptom relief and prevention of disease progression. 1. Superpotent corticosteroids currently provide the best outcomes. However, treatment with ultropotent steroids is recommended for only 4 to 6 weeks.7 3. A typical regimen is a thin layer of steroid applied to the affected area once or twice daily for 2 to 4 weeks, then tapered to three times per week for maintenance therapy.8 Long-term sequelae of potent topical corticosteroids (atrophy and thinning of skin and subcutaneous tissues) have not been clinically significant in this disorder because the vulvar skin is steroid resistant and can tolerate long-term application of superpotent steroids.9 Contact dermatitis is a rare but reported side effect of this medication. 4. Subsequent LS recurrences are managed by reinstating the corticosteroid therapy.9 It may be appropriate to switch to a milder corticosteroid such as betamethasone valerate 1% (high potency) or triamcinolone ointment 0.1% or fluocinolone acetonide ointment 0.025% (moderate potency) for long-term management.10 7. Topical tacrolimus or systemic acitretin or etretinate may be beneficial LS is more common among menopausal women and may exist in the presence of atrophy related to estrogen deficiency. In addition, skin changes associated with aging predispose skin to trauma owing to thinning and decreased elasticity. Thin, nonelastic tissue associated with LS can cause stenosis of the vaginal opening and result in painful intercourse (dyspareunia). This can often be corrected with topical steroids and/or estrogen therapy. A more serious complication is permanent scarring; surgery may be required to lyse adhesions and restore vulvovaginal functioning. If there is indication of atypia, hyperplasia, or a mixed diagnosis on biopsy, a gynecologic referral is indicated, especially in older women. LS is often seen in combination with hyperplasia and requires closer follow-up monitoring.5 Women with LS have a slightly increased risk of developing squamous cell carcinoma. If there is evidence of severe architectural changes and/or vaginal, urethral, or anal stenosis, a surgical consultation is warranted. The role of surgery is defined in LS and should be limited to repair of introital stenosis or confirmed malignant disease.11 Patients who do not respond to a course of topical, high-potency steroids or who report ongoing pain should be referred to a gynecologic dermatology specialist. Other treatment modalities can include oral tricyclic antidepressants (amitriptyline, desipramine, and nortriptyline) and steroid injections. LS requires a long-term treatment plan that will span the lifetime. Women should be educated that this condition is chronic and episodes of symptom control will be interwoven with disease exacerbations. Each woman will respond differently to steroid treatment and it may take some time and different dosing regimens to discover the best treatment plan. Women who are sexually active will need guidance on methods to reduce dyspareunia. Because women with LS have a slightly higher risk of squamous cell carcinoma, the importance of yearly skin checks should be stressed. General patient education information for all women with vaginal and vulvar complaints is listed in Box 170-1. Lichen planus (LP) is an acute or chronic inflammatory dermatosis affecting the skin, scalp, and the mucous membranes of the mouth, vulva, and vagina. The frequency of LP varies according to the population studied, but there is an estimated prevalence of 1% to 2% in the United States.12 LP affects middle-aged people most often, although childhood LP has been described in the literature. Women are affected as often as men.12 The cause is uncertain, but evidence suggests that it may have an autoimmune component, and inflammation precedes the appearance of lesions. Other possible triggers for LP can include infection with the hepatitis C virus and exposure to certain metals and chemicals. In the genital area, there are two presentations of LP: classic or erosive. The appearance of classic LP includes well-defined delicate, white, reticulated papules. Erosive LP involves the presence of erythematous, erosive lesions and a desquamating process. Large denuded areas may lead to profuse leukorrhea or can become adherent, causing stenosis of the vaginal introitus. Physical examination findings can vary with vulvar LP. Erythema is possible, as well as erosions and a lacy appearance on the skin. As with LS, the examination may reveal architectural changes that include loss of genital features, scarring of the vulva and anus, and urethral and vaginal stenosis. Women with LP often complain of a variety of vulvar symptoms, including pain, pruritus, burning, dyspareunia, and dysuria. • Diagnosis is established based on a thorough history and clinical examination findings. • Erosive LP may also have clinical features of herpes simplex virus (HSV) and Bechet disease. • Physical examination is performed to visualize any skin changes. • May be performed with hand-held magnifying glass or colposcope to improve visualization of the skin. • Other diagnostics to consider include the following: • Viral culture for HSV if erosive presentation Because LP may have two different clinical presentations, establishing a correct diagnosis can be challenging. As with LS, this is a chronic condition that requires long-term treatment. Symptoms with vary depending on presentation (classic versus erosive), and women should be prepared for the chronicity of the disorder and expected length of time to symptom improvement. The primary goals of treatment are symptom relief and prevention of disease progression. LP requires lifelong management and treatment that is similar to that for LS. 1. Superpotent corticosteroids are the first-line treatment. 2. Clobetasol, halobetasol, or betamethasone dipropionate augmented 0.05% in an ointment base is recommended. A typical regimen is a thin layer of steroid applied to the affected area once or twice daily for 2 to 4 weeks, then tapered to three times per week for maintenance therapy.8 Long-term sequelae of potent topical corticosteroids (atrophy and thinning of skin and subcutaneous tissues) have not been clinically significant in this disorder because the vulvar skin is steroid resistant and can tolerate long-term application of superpotent steroids.9 Contact dermatitis is a rare but reported side effect of this medication. 7. Petrolatum ointment may be applied as a barrier for sensitive skin. LP often manifests at midlife. For women this often coincides with the perimenopausal and postmenopausal years. LP may exist in the presence of atrophy related to estrogen deficiency, which can further increase discomfort. In addition, skin changes associated with aging predispose skin to trauma as a result of thinning, decreased elasticity, and a decreased ability to act as a first-line protection against injury. Severe LP can cause significant pain, decreased quality of life, and interference with daily activities. Thin, nonelastic tissue associated with classic LP can cause stenosis of the vaginal opening and result in painful intercourse. This can often be corrected with topical steroids and/or estrogen therapy. A more serious complication is permanent scarring; surgery may be required to lyse adhesions and restore vulvovaginal functioning. Erosive LP can cause chronic pain, sexual dysfunction, and dysuria. If there is indication of atypia, hyperplasia, or a mixed diagnosis on biopsy, a gynecologic referral is indicated, especially in older women. • If there is evidence of severe architectural changes and/or vaginal, urethral, or anal stenosis, a surgical consultation is warranted. The role of surgery is limited; surgery is used only to repair introital stenosis or confirmed malignant disease.11 • Patients who do not respond to a course of topical, high-potency steroids or who report ongoing pain should be referred to a gynecologic dermatology specialist and possibly a pain management specialist. Other treatment modalities can include topical application of an immunomodulating medication (tacrolimus)13 and intramuscular injection of triamcinolone acetate.14 Similar to LS, LP is a chronic condition that requires treatment over the life span. Symptoms and subsequent treatment will vary depending on whether the woman has classic or erosive LP. Pain is common with LP and can significantly affect a woman’s quality of life and sexual functioning. Symptom management may involve a multidisciplinary approach that includes gynecologists, dermatologists, pain specialists, and pelvic floor physical therapy. The use of vaginal dilators may be necessary to prevent scarring and stenosis of the vaginal introitus. Women with LP should receive education on general vulvar care, including loose-fitting clothing; mild soaps; gentle patting of the vulvar area to dry; mild laundry detergent; cotton underwear; and sleeping without underwear or pajama bottoms. See Box 170-1. Lichen simplex chronicus (LSC), or squamous cell hyperplasia, is the result of repetitive surface trauma from irritants that cause scratching or rubbing—a perpetual itch-scratch cycle. LSC is characterized histologically by epithelial thickening, lichenification, hyperkeratosis, and eczematous inflammation. It manifests, as do most of the non-neoplastic epithelial disorders, as pruritus, which may be secondary to degeneration and inflammation of terminal nerve fibers. The cause is not completely known, but atopic dermatitis is a common finding in women affected by LSC. Intense itching (vulvar pruritus) is often the chief complaint. The pruritus may be aggravated by friction, heat, perspiration, or tight clothing. The affected woman may complain of a history of chronic scratching, which causes further pruritus. The pruritus often occurs at night and therefore sleep disturbances and fatigue are common. Painful erosions and fissures can result from the chronic scratching. LSC may affect the labia majora, outer aspect of the labia minora, and anal area. On examination, the skin may exhibit thickening, and fissures are possible. Often, LSC initially manifests with small red papules. Reddened plaques develop from the papules, or if the condition has been ongoing without treatment, the skin may be more deeply colored, rough, and furrowed. Areas of excoriation are common.10 Because LSC has a presentation and symptoms similar to those of many other pruritic vulvar conditions, it is important to establish a correct diagnosis. Treatment for LSC focuses on interrupting the itch-scratch cycle. Treatment is aimed at relief of the itch-scratch cycle. 3. Removing any potential skin irritants is imperative. 4. Thickened plaques can be soaked in warm water or 5% Burow solution.1 After thorough drying, an emollient such as petrolatum can be applied as a skin barrier. 6. Tricyclic antidepressants such as amitriptyline taken at bedtime will also help control pruritus. 7. Screen for secondary fungal and bacterial skin infections. 8. Women should be reevaluated 4 to 8 weeks after beginning treatment. Although LSC can occur in children, it most often occurs after age 30 and can coincide with the menopausal transition. This is a chronic condition that women will need to manage throughout the midlife and older adult years. Aging skin is more susceptible to trauma from scratching, and advising patients to wear soft cotton gloves to bed may help reduce skin breakdown from scratching. As women age, urinary incontinence is more common, and wet skin from exposure to urine increases the risk of excoriation and infection. LSC in the presence of atrophy from estrogen deficiency of menopause may need to be treated with topical estrogen therapy. LSC can cause skin changes such as hypopigmentation or hyperpigmentation that may be permanent. The severe pruritus that is a hallmark symptom of LSC can cause sleep disturbances, fatigue, and decreased quality of life. Skin breakdown places patients at risk for superimposed bacterial and fungal infections that can be painful. Decreased sexual function from pruritus and pain is possible. If there is indication of atypia, hyperplasia, or a mixed diagnosis on biopsy, a gynecologic referral is indicated, especially in older women. Similar to LS and LP, LSC is a chronic condition that requires treatment over the life span. Tricyclic antidepressants at bedtime are often prescribed to help control itching and scratching symptoms and not to control depressive symptoms. Women need education regarding their treatment plan and the multifaceted approach to the management of this disorder. Contact dermatitis of the vulvovaginal area results when the area comes in contact with an allergen or irritant. This causes localized inflammation and edema. Pruritus is common. Contact dermatitis occurs when there is an immediate irritation of the area after exposure to an offending substance. Allergic responses, on the other hand, develop several days after exposure.10 Women with vulvar contact dermatitis often report a recent onset of vulvar irritation, pruritus, burning, soreness, or discomfort. These symptoms often worsen with friction or if skin is wet. Dysuria may result when acidic urine passes over inflamed or excoriated skin. On examination, erythema is typically present, and excoriation can result from scratching. Skin may be thickened if contact with the irritant or allergen has occurred over an extended period of time. Vaginal discharge is not common. Vulvar pruritus, irritation, burning, and pain are associated with a variety of vulvar conditions. Although erythema and edema are hallmark symptoms of contact dermatitis, other vulvar conditions must be considered. • The goal of management is identifying the triggering substance and eliminating it. • The list of possible irritants is extensive (Box 170-2). Women should be asked about treatments to the vulva (either prescribed or OTC medications) and use of feminine hygiene products (such as douches, sprays, and deodorants), tampons or pads, condoms, spermicides, lubricants, laundry detergents, soaps, and shampoos.
Vulvar and Vaginal Disorders
Vulvar Disorders
Definition and Epidemiology
Lichen Sclerosus
Pathophysiology
Clinical Presentation and Physical Examination
Diagnostics
Differential Diagnosis
Management
Specialist referral is indicated for biopsy results that indicate hyperplasia or atypia. Surgical consultation is warranted if there is evidence of severe architectural changes or scarring. Women with LS have a slightly increased risk of developing squamous cell carcinoma. If there is evidence of severe architectural changes and/or vaginal, urethral, or anal stenosis, a surgical consultation is warranted.
Life Span Considerations
Complications
Indications for Referral or Hospitalization
Patient and Family Education
Lichen Planus
Pathophysiology
Clinical Presentation and Physical Examination
Diagnostics
Differential Diagnosis
Management
Specialist referral is indicated for biopsy results that indicate hyperplasia or atypia. Surgical consultation is warranted if there is evidence of severe architectural changes or scarring.
Life Span Considerations
Complications
Indications for Referral or Hospitalization
Patient and Family Education
Lichen Simplex Chronicus
Pathophysiology
Clinical Presentation and Physical Examination
Differential Diagnosis
Management
Specialist referral is indicated for biopsy results that indicate atypia.
Life Span Considerations
Complications
Indications for Referral or Hospitalization
Patient and Family Education
Contact Dermatitis
Pathophysiology
Clinical Presentation and Physical Examination
Differential Diagnosis
Management
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